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Elevated serum prolactin concentrations in phenylketonuric patients on a 'loose diet'.
[classical phenylketonuria]
High
levels
of
phenylalanine
(
Phe
)
have
been
shown
to
reduce
dopamine
(
DA
)
and
noradrenaline
(
NA
)
production
.
We
have
therefore
evaluated
whether
increased
serum
Phe
concentrations
lead
to
increased
prolactin
(
PRL
)
secretion
in
phenylketonuria
.
Forty
-
two
patients
with
classical
phenylketonuria
were
divided
into
two
groups
.
Group
A
(
n
=
19
)
adhered
very
strictly
to
their
special
diet
(
Phe
=
0
.
09
+
/
-
0
.
039
mmol
/
l
)
and
Group
B
(
n
=
23
)
were
on
a
'
loose
diet
'
(
Phe
=
0
.
77
+
/
-
0
.
45
mmol
/
l
)
.
Thirty
healthy
children
of
comparable
age
served
as
controls
.
PRL
was
measured
by
RIA
,
and
Phe
and
tyrosine
(
Tyr
)
with
an
amino-acid
autoanalyser
.
Adrenaline
(
A
)
,
noradrenaline
(
NA
)
,
and
dopamine
(
DA
)
were
measured
by
HPLC
.
Serum
PRL
in
group
B
patients
(
1595
+
/
-
57
mU
/
l
)
was
significantly
raised
compared
to
Group
A
patients
(
243
+
/
-
57
mU
/
l
)
and
controls
(
201
+
/
-
55
mU
/
l
)
(
P
<
0
.
001
)
.
Serum
DA
,
A
and
NA
in
group
B
were
lower
than
in
group
A
patients
and
controls
.
Serum
Phe
in
group
B
patients
was
significantly
correlated
to
serum
PRL
(
r
=
0
.
59
)
and
DA
(
r
=
-
0
.
41
)
.
Irregular
menses
were
observed
in
1
/
10
women
from
group
A
but
in
10
/
13
in
group
B
.
High
serum
phenylalanine
concentrations
in
phenylketonuric
patients
not
strictly
adhering
to
their
diets
are
correlated
with
high
serum
PRL
and
low
serum
dopamine
concentrations
,
and
a
high
prevalence
of
menstrual
irregularities
.
Diseases
Validation
Diseases presenting
"high levels"
symptom
22q11.2 deletion syndrome
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
alpha-thalassemia
aromatase deficiency
cadasil
canavan disease
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cutaneous mastocytosis
cystinuria
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
erythropoietic protoporphyria
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
holt-oram syndrome
homocystinuria without methylmalonic aciduria
kabuki syndrome
kallmann syndrome
liposarcoma
papillon-lefèvre syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
scrub typhus
severe combined immunodeficiency
systemic capillary leak syndrome
triple a syndrome
von hippel-lindau disease
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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